A case of vasa previa in twin pregnancy associated with a sinusoidal heart rate was observed. A review of the world literature revealed eight previously reported cases of vasa previa in twins. No first twin survived, and 62.5% of second twins eventually died from partial or complete exsanguination. This is the first reported case of sinusoidal fetal heart rate in association with vasa previa. Continuous fetal monitoring suggested the diagnosis of funic presentation and of fetal bleeding. A high index of suspicion, use of amnioscopy, ability to detect fetal blood in the vaginal pool, continuous fetal heart rate monitoring and ultrasonography may help in reducing the high perinatal mortality associated with vasa previa.